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Lymphangiomyomatosis and Tuberous Sclerosis
- Similar in pathology and x-ray
appearance
- Widespread proliferation of smooth
muscle in pleura, alveolar septa, bronchi, pulmonary vessels and
lymphatics as well as lymph nodes, especially in posterior mediastinum and
retroperitoneum
- Focal emphysema develops as result
of narrowing of airways
- Thoracic duct may be obliterated
- Produce multiple small cysts with a
hamartomatous proliferation of smooth muscle in their walls
Characteristic
imaging triad of:
- Progressive, diffuse interstitial
disease
- Recurrent chylous effusions and
sometimes chylous ascites
- Recurrent pneumothorax
- Tuberous
sclerosis is
inherited as a dominant with variable penetrance:
o
Mental defects
o
Epilepsy
o
Retinal phacoma
o
Angiomyolipomas of the kidneys
o
Rhabdomyomas of the heart
o
Intracranial calcifications
o
Sclerotic skull lesions
o
Adenoma sebaceum
o
Subungual fibromas
o
Pulmonary
lymphangiomyomatosis (syn:pulmonary
myomatosis)
§
Exclusively
in females ages 17-47 years
§
Rare
Imaging findings
- Identical in both tuberous sclerosis
and lymphangiomyomatosis and indistinguishable from pulmonary fibrosis
except for decreased lung volume in fibrosis and increased lung volume in
the others
- CT
- Coarse,
reticular interstitial pattern
- Normal/increased
lung volume
- Numerous
thin-walled pulmonary cysts and honeycombing
- Various
sizes/surrounded by normal lung parenchyma
·
Unilateral
or bilateral pleural effusions which are usually large and recurrent
·
Spontaneous
pneumothorax is common
Clinically
- Progressive
exertional dyspnea and cough
- Hemoptysis
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